GCH1 抗体
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北京 101111
Quick Overview for GCH1 抗体 (ABIN7228628)
抗原
See all GCH1 抗体适用
宿主
克隆类型
标记
应用范围
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原理
- Rabbit Anti-GCH1 Polyclonal Antibody
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特异性
- The antibody detects endogenous levels of GCH1 protein
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纯化方法
- The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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免疫原
- Synthesized peptide derived from part region of human GCH1 protein
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亚型
- IgG
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应用备注
- Optimal working dilutions should be determined experimentally by the investigator. Suggested starting dilutions are as follows: WB 1:500-2000,ELISA 1:5000-20000
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限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 mg/mL
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缓冲液
- PBS, 50 % glycerol, 0.05 % Proclin 300, 0.05 %BSA
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Stable for one year at -20°C from date of shipment. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Aliquot to avoid repeated freezing and thawing.
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有效期
- 12 months
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- GCH1 (GTP Cyclohydrolase 1 (GCH1))
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别名
- GCH1
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背景
- GTP cyclohydrolase 1, GTP cyclohydrolase I, GTP-CH-IGCH1 encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin (BH4) biosynthesis, catalyzing the conversion of GTP into 7,8-dihydroneopterin triphosphate. BH4 is an essential cofactor required by aromatic amino acid hydroxylases as well as nitric oxide synthases. Mutations in this gene are associated with malignant hyperphenylalaninemia and dopa-responsive dystonia. Several alternatively spliced transcript variants encoding different isoforms have been described, however, not all variants give rise to a functional enzyme. GCH1 (GTP Cyclohydrolase 1) is a Protein Coding gene. Diseases associated with GCH1 include Hyperphenylalaninemia, Bh4-Deficient, B and Dystonia, Dopa-Responsive, With Or Without Hyperphenylalaninemia. Among its related pathways are eNOS activation and regulation and Metabolism.
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分子量
- 27kD
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基因ID
- 2643
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UniProt
- P30793
抗原
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